Myelofibrosis is a rare type of blood cancer in which the bone marrow is replaced with scar tissue. Bone marrow is the soft spongy tissue present inside the bones which contain immature cells that later develop into our blood cells – red blood cells, platelets, and white blood cells. Myelofibrosis occurs when the bone marrow cells develop changes (mutation) in the DNA. When the defective cell divides, the new cells produced will carry the change.
As time goes by, more abnormal cells will be produced and replace the bone marrow tissue, and it will lose its ability to produce healthy blood cells. Researchers still do not know why the mutations occur, and several gene mutations are observed in people with myelofibrosis. Around fifty percent of the people with myelofibrosis have the mutation in the Janus associated kinases (JAK) gene. Although myelofibrosis can affect anyone, it is most commonly diagnosed in people above the age of 50. Exposure to industrial chemicals such as toluene, exposure to high doses of radiation and certain disorders can increase the risk of developing myelofibrosis.
When this disorder occurs on its own, it is called primary myelofibrosis, and if it occurs as a result of other disorders called essential thrombocythemia (excessive production of platelets) or polycythemia vera (excessive production of red blood cells), it is called secondary myelofibrosis.
Myelofibrosis is a slow-growing cancer, and most people do not have symptoms in their early stages and even for many years. When the abnormal cells continue to grow, the patients may experience bleeding or bruising easily, excessive sweating at night, bone pain, fever, anemia (shortness of breath, tiredness), and pain below the ribs on the left side caused by an enlarged spleen.
Bone marrow biopsy confirms the diagnosis of myelofibrosis, in which your health care professional collects bone marrow tissue samples and analyzes them in the laboratory. Imaging tests such as MRI will provide much information on your myelofibrosis. Your doctor may do some laboratory tests to find out if your cancer cells contain a specific gene mutation which helps formulate the treatment and prognosis.
In general, if the patient does not have an enlarged spleen or anemia or has very mild anemia, he/ she may not need treatment. If you have moderate to high-risk myelofibrosis, treatment options will be recommended to manage the symptoms. Bone marrow transplantation may offer a cure for some patients, but it is not suitable for all and carries a certain risk of complications. For anemia, blood transfusions, androgen therapy, and thalidomide & related medications can help manage the condition.
For enlarged spleen, the doctor may suggest surgical removal of the spleen, chemotherapy, targeted therapy, or radiation therapy. Ruxolitinib is the targeted therapy available to treat people with moderate to high-risk myelofibrosis. This medication is also used in the treatment of polycythemia vera and graft-versus-host-disease (a complication that occurs after the transplantation when donor cells begin attacking host cells). Ruxolitinib works by blocking the action of enzymes called Janus associated kinases and reduces the symptoms.
Jakavi 5mg Tablet is one of the products available in the market containing the active substance Ruxolitinib. Your physician may prescribe Jakavi 5mg Tablet if you have any of the above disorders.
